Difficult airway in a patient with Marshall‐Smith syndrome
Difficult airway in a patient with Marshall‐Smith syndrome
复制标题
马歇尔-史密斯综合征患者气道困难
DOI：
10.1046/j.1460-9592.1998.00763.x
复制
发表时间：
1998
影响因子：
1.7
通讯作者：
Pakkanen
中科院分区：
文献类型：
--
作者：
Antila;Laitio;Aantaa;Silvoniemi;Pakkanen
马歇尔-史密斯综合征是一种罕见的临床疾病，其特征是骨成熟加速、面部特征畸形、气道异常以及婴儿早期因呼吸系统并发症而死亡。尽管马歇尔-史密斯综合征患者有一些潜在麻醉问题的特征，但之前不存在关于这些患者麻醉管理的报道。我们介绍了一个病例，一名患有这种综合征的八个月大男婴在常规麻醉诱导后迅速出现严重缺氧。经过多次尝试均未成功，最终通过盲探口腔插管确保了气道的安全。两周后，用纤维喉镜检查喉部解剖结构，发现明显的喉软化症。进行喉镜检查时，氯胺酮麻醉和自主呼吸没有出现任何问题。对马歇尔-史密斯综合征患者进行麻醉时，应始终牢记气道受损的可能性。对于患有这种综合征的儿童来说，维持自主呼吸的麻醉是最安全的。如果需要气管插管或肌肉放松，则需要采取预防措施以保持呼吸道通畅。插管前应避免使用肌肉松弛剂。
Marshall‐Smith syndrome is a rare clinical disorder characterized by accelerated bone maturation, dysmorphic facial features, airway abnormalities and death in early infancy because of respiratory complications. Although patients with Marshall‐Smith syndrome have several features with potential anaesthetic problems, previous reports about anaesthetic management of these patients do not exist. We present a case, in which severe hypoxia developed rapidly after routine anaesthesia induction in an eight‐month‐old male infant with this syndrome. After several unsuccessful attempts the airway was finally secured by blind oral intubation. After 2 weeks, laryngeal anatomy was examined with fibreoptic laryngoscopy which revealed significant laryngomalacia. Laryngoscopy was performed without problems with ketamine anaesthesia and spontaneous breathing. The possibility of a compromised airway should always be borne in mind when anaesthetizing patients with Marshall‐Smith syndrome. Anaesthesia maintaining spontaneous breathing is safest for children with this syndrome. If tracheal intubation or muscle relaxation is required, precautions are needed to maintain a patent airway. Muscle relaxants should possibly be avoided before intubation.